上海交通大学学报（医学版）

上海交通大学学报（医学版）

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外周型原始神经外胚层瘤临床病理分析

齐 琦1，郭 飞2，武世伍3   

  1. 1.蚌埠医学院组织胚胎学教研室， 蚌埠 233030;  2.蚌埠医学院第一附属医院影像科， 蚌埠 233004; 3.蚌埠医学院第一附属医院病理科， 蚌埠 233004
  • 出版日期:2015-01-28 发布日期:2015-01-29
  • 作者简介:齐 琦（1977—）， 女， 讲师， 硕士; 电子信箱: houniao1234567@sina.com。
  • 基金资助:

    蚌埠医学院省级实验室（工程中心）开放课题（BYKL1201）；蚌埠医学院科研（自然科学类）课题（BYKY1321）

Clinicopathologic analysis of peripheral primitive neuroectodermal tumors

QI Qi1, GUO Fei2, WU Shi-wu3   

  1. 1.Department of Histology and Embryology, Bengbu Medical College, Bengbu 233030, China; 2.Department of Radiology, the First Affiliated Hospital of Bengbu Medical College, Bengbu 233004, China; 3.Department of Pathology, the First Affiliated Hospital of Bengbu Medical College, Bengbu 233004, China
  • Online:2015-01-28 Published:2015-01-29
  • Supported by:

    Open Project of Provincial Level Laboratory, Engineering Center, of Bengbu Medical College, BYKL1201; Scientific Research Project (Natural Science) of Bengbu Medical College, BYKY1321

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摘要：

目的 探讨外周型原始神经外胚层瘤（pPNET)的临床病理及免疫组织化学特点和诊断标准。方法 采用常规苏木精-伊红染色，免疫组织化学法，结合影像学表现，对12例pPNET进行观察分析。结果 12例患者临床表现为增长迅速的局部肿块，伴有疼痛，以及肿块压迫所引起的症状。影像学表现缺乏特异性。镜下肿瘤主要由小圆细胞构成，3例可见Homer-Wright 菊形团形成；免疫组织化学结果显示CD99阳性率为100%（12/12），NSE和Syn阳性率较高，分别为92%(11/12)和75%(9/12)，Vim的阳性表达率为50%(6/12)。结论 pPNET 是一种少见的高度恶性小圆细胞肿瘤，病理形态学特征及免疫组织化学标记有助于pPNET的诊断和鉴别诊断。

关键词: 外周型原始神经外胚层瘤, 病理, 组织, 诊断

Abstract:

Objective To investigate the clinicopathologic and immunohistochemical characteristics of peripheral primitive neuroectodermal tumors (pPNET) and its diagnostic criteria. Methods 12 cases of pPNET were observed and analyzed by the HE staining, immmunohistochemistry, and imaging examination. Results 12 patients with pPNET presented progressively growing masses, local pain, and symptoms caused by mass compression. Imaging findings lacked specificity. Under the microscope, tumors consisted of small round cells and Homer-Wright rosettes were observed in 3 cases. Results of immunohistochemistry showed that the positive rate of CD99 was 100% (12/12). Positive rates of NSE and Syn were high, i.e. 92% (11/12) and 75%(9/12), respectively. The positive rate of Vim was 50% (6/12). Conclusion pPNET is a rare, highly malignant sarcoma. Pathological and morphological characteristics and immunohistochemistry labels are helpful for the diagnosis and differential diagnosis of pPNET.

Key words: peripheral primitive neuroectodermal tumors, pathology, tissue, diagnosis